Advances in Therapy

, Volume 36, Issue 5, pp 1221–1232 | Cite as

Long-Term Effects and Adverse Events of Nintedanib Therapy in Idiopathic Pulmonary Fibrosis Patients with Functionally Advanced Disease

  • Eniko Barczi
  • Livia Starobinski
  • Abigel Kolonics-Farkas
  • Noemi Eszes
  • Aniko Bohacs
  • Martina Vasakova
  • Karel Hejduk
  • Veronika MüllerEmail author
Brief Report



Idiopathic pulmonary fibrosis (IPF) is one of the most common interstitial lung diseases with limited survival. The effect of IPF therapy in patients with severely impaired lung function is not well established. The aim of this study was to characterize IPF patients with a forced vital capacity (FVC) < 50% (group 1) and FVC 50–60% predicted (group 2) and analyze the effect and adverse events of nintedanib in Hungarian patients diagnosed between April 2015 and July 2017.


The impact of nintedanib therapy on lung function, survival, and adverse events was analyzed longitudinally.


Twenty-two out of 103 patients were included in the analysis (group 1: N = 10; male/female = 6:4, age 62.6 ± 10.8 years and group 2: N = 12; male/female = 3:9, age 65.7 ± 11.6 years). Eighteen patients were treated with nintedanib (8 in group 1, 10 in group 2); treatment stabilized lung function in 42% and 50%, respectively, in the two groups. Median survival was 444 days for group 1 and 476 days for group 2. Adverse events were less common than in clinical trials; dose reduction was necessary in three cases, drug discontinuation in two cases. No differences between groups were identified regarding clinical parameters and radiological pattern; however, hypertension as comorbidity was more common in group 1 patients.


Nintedanib therapy was effective and well tolerated even among patients with severely impaired lung function. Longitudinal follow-up confirmed high mortality in patients with very severe and severe IPF; however, median survival was meaningful as it exceeded 1 year in both groups.


IPF Nintedanib Pharmacotherapy Respiratory/pulmonary Safety Severe IPF Survival 



The authors would like to thank all the members of EMPIRE registry.


The EMPIRE registry is supported by a grant from Boehringer Ingelheim (since 2014). Eniko Barczi is supported by “Development of scientific workshops of medical, health sciences and pharmaceutical educations” (EFOP-3.6.3-VEKOP-16-2017-00009). Article processing charges were funded by Masaryk University. All authors had full access to all of the data in this study and took complete responsibility for the integrity of the data and accuracy of the data analysis.


All named authors meet the International Committee of Medical Journal Editors (ICMJE) criteria for authorship for this article, take responsibility for the integrity of the work as a whole, and have given their approval for this version to be published.


Veronika Müller received consultation fees or travel grants from Boehringer Ingelheim and Roche. Aniko Bohacs received consultation fees or travel grants from Boehringer Ingelheim and Roche. Noemi Eszes received consultation fees or travel grants from Boehringer Ingelheim and Roche. Eniko Barczi, Livia Starobinski, Abigel Kolonics-Farkas, Martina Vasakova, and Karel Hejduk declare having nothing to disclose.

Compliance with Ethics Guidelines

The study protocol was approved by the Ethical Committee of Semmelweis University. Regional and Institutional Committee of Science and Research Ethics (TUKEB 69/2015) in accordance with the Declaration of Helsinki. Additional informed consent was obtained from all individual participants for whom identifying information is included in this article.

Data Availability

The datasets generated during and/or analyzed during the current study are available from the corresponding author on reasonable request.


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Copyright information

© Springer Healthcare Ltd., part of Springer Nature 2019

Authors and Affiliations

  • Eniko Barczi
    • 1
  • Livia Starobinski
    • 1
  • Abigel Kolonics-Farkas
    • 1
  • Noemi Eszes
    • 1
  • Aniko Bohacs
    • 1
  • Martina Vasakova
    • 2
  • Karel Hejduk
    • 3
  • Veronika Müller
    • 1
    Email author
  1. 1.Department of PulmonologySemmelweis UniversityBudapestHungary
  2. 2.Department of Respiratory Diseases of the First Faculty of MedicineCharles University, Thomayer HospitalPragueCzech Republic
  3. 3.Masaryk UniversityBrnoCzech Republic

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